Persistent fifth aortic arch: a comprehensive literature review

Abstract

Persistent fifth aortic arch (PFAA) is an extremely rare congenital cardiovascular anomaly resulting from the failure of the fifth aortic arch to degenerate during embryonic development; it is often associated with various other cardiovascular anomalies. Despite being first reported by Van Praagh in 1969, there have been only a few individual case reports. Owing to its rarity and lack of comprehensive understanding, PFAA is often misdiagnosed or missed diagnosed during clinical. Thus, this review aimed to summarise the embryonic development, pathological classification, imaging diagnosis, and clinical treatment of PFAA to improve its overall understanding, ultimately helping in accurate diagnosis and treatment.

Keywords: classification; diagnosis; imaging; persistent fifth aortic arch; treatment.

Figure 1

The developmental process of each pair of aortic arches in the embryonic stage. (A) Each pair of arterial arches connects to the ventral aorta and dorsal aorta; (B) a simple graph of the development of each pair of arterial arches in the embryo.

Figure 2

Diagram of Weinberg classification (5). (A) Weinberg type A, double-lumen aortic arch; (B) Weinberg type B, single-lumen aortic arch; (C) Weinberg type C, pulmonary to descending aorta.

Figure 3

Diagram of improved classification (7). Type A, systemic-to-systemic connection; Type B, systemic-to-pulmonary connection; Type C, pulmonary-to-systemic connection; Type D, Bilateral PFAA, with right double-lumen aortic arch and left PFAA. Ao, ascending aortic arch; IV, aortic arch; V: fifth aortic arch; PA, pulmonary artery; RPA, right pulmonary artery; and VI, sixth branchial arch (arterial ducts).